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Agenesis of the dorsal pancreas

Other Names for this Disease
  • Complete agenesis of the dorsal pancreas
  • Congenital short pancreas
  • Pancreas agenesis, dorsal
  • Pancreas, dorsal, agenesis of
  • Partial agenesis of the dorsal pancreas
More Names
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Overview


Agenesis of the dorsal pancreas describes a congenital malformation of the pancreas in which either the entire dorsal pancreas or part of the dorsal pancreas fails to develop (complete agenesis or partial agenesis, respectively). Some individuals experience no symptoms, while others may develop hyperglycemia, diabetes mellitus, bile duct obstruction, abdominal pain, pancreatitis, or other conditions.[1][2][3] Hyperglycemia has been shown to be present in approximately 50% of affected individuals.[3] The cause of agenesis of the dorsal pancreas is currently not well understood. It may occur in individuals with no history of the condition in the family (sporadically) and in some cases, autosomal dominant or X-linked dominant inheritance has been suggested.[4][5] It has also been reported to occur with very rare conditions including polysplenia and polysplenia/heterotaxy syndrome.[6]


References

  1. Whitcomb DC, Lowe ME. Hereditary, Familial, and Genetic Disorders of the Pancreas and Pancreatic Disorders in Childhood. In: Feldman. Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 9th ed. Philadelphia, PA: Saunders; 2010.
  2. Schnedl WJ, Piswanger-Soelkner C, Wallner SJ, Reittner P, Krause R, Lipp RW, Hohmeier HE. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci. 2009 Mar.
  3. Wolfgang J Schnedl, Claudia Piswanger-Soelkner, Sandra J Wallner, Robert Krause, Rainer W Lipp. Agenesis of the dorsal pancreas. World Journal of Gastroenterology. January 2009. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2653338/pdf/WJG-15-376.pdf. Accessed July 22, 2011.
  4. Joanna S. Amberger . Pancreas, Dorsal, Agenesis of. OMIM. http://omim.org/entry/167755. Accessed July 22, 2011.
  5. Joo YE, Kang HC, Kim HS, Choi SK, Rew JS, Chung MY, Kim SJ. Agenesis of the dorsal pancreas: a case report and review of the literature. Korean J Intern Med. December 2006. http://www.kjim.or.kr/upload/abstract/files/062104236.pdf. Accessed July 22, 2011.
  6. Suraj Kapa, Ferga C Gleeson, Santhi Swaroop Vege. Dorsal Pancreas Agenesis and Polysplenia/Heterotaxy Syndrome: A Novel Association with Aortic Coarctation and a Review of the Literature. JOP. J Pancreas (Online). 2007. http://www.joplink.net/prev/200707/200707_02.pdf. Accessed July 22, 2011.
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General Information

  • The Human Embryology: Organogenesis online text book provides a detailed description and illustrations of pancreas development. Click on the link above to view this description.
  • The United Network for Organ Sharing Web site provides information on the pancreas in general. Click on the link above to view the information page.
  • The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) conducts and supports research on many of the most serious diseases affecting public health. Click on the link for more information on pancreatitis.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.  Click on the link to read information on this topic.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Agenesis of the dorsal pancreas. Click on the link to view a sample search on this topic.
  • The The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Agenesis of the dorsal pancreas. Click on the link to go to OMIM and review these resources.

Selected Full-Text Journal Articles